Identify the Disorder

A 25 year old man was at work and had a syncopal episode.  At the behest of his boss, he has come to the ED for evaluation.  The patient feels fine now and wants to go home.  The following EKG was obtained. 

Please identify the disorder and the subsequent treatment.

Answer: Brugada Syndrome

High Yield Points

- Brugada syndrome can cause polymorphic ventricular tachycardia leading to syncope, cardiac arrest or sudden death.

- Condition an example of a channelopathy.  10-30% of cases linked to mutations of cardiac voltage-gated sodium channels.

- Autosomal dominant pattern of transmission in about 50% of familial cases.

- Typical patient young, male, and otherwise healthy, with normal general medical and cardiovascular physical examinations.

- Per eMedicine, Implantable cardioverters-defibrillators (ICDs) are often used to treat patients with Brugada syndrome, decision can be made in concert with Cardiology.  No pharmacologic therapy has been proven to reduce the occurrence of ventricular arrhythmias or sudden death.

EKG Manifestations - Slightly different for Type I, II, or III

Generally speaking - RBBB with ST elevations in V1-V2 (some sources also claim ST elevations in V3 as well).

Only Type I is diagnostic of Burgada given the correct clinical setting and/or family risk factors.

The type I ECG is characterized by a J elevation >=2 mm (0.2 mV) a coved type ST segment followed by a negative T wave.